Juvenile xanthogranulomas in Asian children.

Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder. It can rarely be associated with systemic involvement. There is a paucity of literature on JXG in Asian children. We aim to describe the epidemiology, clinical features, systemic associations, histological features and outcome of a cohort of Asian children with JXG, and review the literature on the condition. We retrospectively reviewed the demographic, clinical and histological data of patients less than 16 years of age, diagnosed with JXG at our tertiary pediatric hospital between January 2002 and April 2019. A total of 147 children with JXG were identified, with a slight male preponderance of 53.1%. The median age of the onset was 15.5 months, with 69.4% presenting before 2 years of age. There was no racial predilection. The most frequently involved site was the head and neck region (44.2%). The majority of patients (76.2%) presented with a solitary lesion. Spontaneous resolution was documented in 57.7% of our patients with mean duration to resolution of 18.8 months. The proportion and speed of resolution did not differ in children with single or multiple lesions. No ophthalmologic complications were detected in our study cohort. JXG in children is generally limited to the skin and is rarely associated with systemic involvement, including the eye. Unless clinically indicated, the results from our study does not support routine screening for juvenile myelomonocytic leukemia, eye or systemic complications, even in the setting of multiple cutaneous JXGs.

Moisturisers from birth in at-risk infants of atopic dermatitis - a pragmatic randomised controlled trial.

BACKGROUND: Atopic dermatitis (AD) is a common, chronic dermatosis, with onset of disease often manifesting in early infancy. Past studies evaluating the early use of moisturisers in the prevention of AD had mixed results. OBJECTIVES: To compare the incidence of moderate or severe AD and total incidence of AD in a cohort of 'at-risk' infants treated with moisturisers from the first 2 weeks of life, to a similar group without moisturisers. METHODS: We performed a single-centre, prospective, parallel-group, randomised study in infants with at least 2 first-degree relatives with atopy. Subjects were randomised into either a treatment group with moisturisers or a control group without moisturisers. Participants were assessed at 2, 6, and 12 months for AD and if present, the severity was assessed using SCORAD index. We also compared the overall incidence of AD, trans-epidermal water loss (TEWL), stratum corneum (SC) hydration, pH, and incidence of food and environmental sensitisation and allergies between both groups. Genotyping for loss-of-functions mutations in the FLG gene was conducted. RESULTS: A total of 200 subjects were recruited, with 100 subjects in each arm. There was no significant difference in incidence of moderate or severe AD, and total incidence of AD at 12 months between the treatment and control groups. There was a lower mean SCORAD in the treatment group than in the control group, but no significant difference in TEWL, SC hydration, and skin pH. No significant side-effects were reported. CONCLUSIONS: The early use of moisturisers in 'at-risk' infants does not reduce the incidence of moderate-to-severe AD and overall incidence of AD in infancy.

Comparing the Potential for Irritation of a Ceramide-Based Moisturizer with a Urea-Based Moisturizer for Pediatric Atopic Dermatitis.

INTRODUCTION: Moisturizers are one of the mainstays of the topical treatment of atopic dermatitis (AD). One of the adverse effects of moisturizers is skin irritation, especially on excoriated AD skin. We compared the potential for irritation of two commercially available moisturizer products for the treatment of AD: a ceramide-based moisturizer (Ceradan® Cream; Hyphens Pharma Pte Ltd, Singapore) and a urea 5% moisturizer (Aqurea Lite Cream; ICA Pharma Pte Ltd, Singapore). METHODS: We performed a prospective single-blind randomized controlled study recruiting AD patients aged between 8 and 16 years with symmetrical or near symmetrical scratch marks (excoriations) of at least grade 2 to 3 severity score, according to the Eczema Area and Severity Index (EASI), over bilateral antecubital fossae. Subjects were randomized to receive the ceramide-based moisturizer to either the left or right antecubital fossa or urea 5% cream to the other antecubital fossa. Subjects were asked to grade the immediate skin irritation of both creams on a standard visual analogue scale (VAS) and which cream they would prefer to use as a daily moisturizer. Primary outcome was the mean irritant score of each cream, and secondary outcome was the subjects' preference of either cream as their daily moisturizer. RESULTS: A total of 42 participants were enrolled with a mean age of 11 years 5 months. The ceramide-based cream had a significantly lower mean VAS score (mean 0.69, SD = 1.63) for irritation compared with urea 5% cream (1.43, SD = 1.64) (p = 0.035). More participants also preferred the ceramide-based cream over urea 5% cream (62% versus 38%) as their daily moisturizer, but this did not reach statistical significance (p = 0.164). CONCLUSIONS: A ceramide-based moisturizer may be considered as a suitable choice for children to minimize irritation from moisturizer treatment for AD.

Rumpel-Leede Phenomenon-An alarming rash with benign etiology in 4 healthy infants.

The Rumpel-Leede phenomenon (RLP) is a rare clinical presentation and in some cases shown to be related to serious systemic conditions. In children, it has been described in association with idiopathic thrombocytopenic purpura and Henoch-Schönlein purpura. We present a series of pediatric cases of RLP with mechanical etiologies that had a benign course. We propose minimal investigations for pediatric RLP cases who are systemically well and have a clear suggestive history of a mechanical cause.

Vulvar lymphangiectasia secondary to gastrointestinal tuberculosis infection in a teenager.

We report a rare presentation of vulvar lymphangiectasia that developed secondary to gastrointestinal tuberculosis in a teenager, and its spontaneous resolution after anti-tuberculous treatment was completed.

Factors influencing quality of life in children with atopic dermatitis and their caregivers: a cross-sectional study.

Better understanding of atopic dermatitis' effect on quality of life could enhance current management and therapeutic strategies. Studies investigating factors related to the health-related quality of life (HRQOL) of children with atopic dermatitis and their caregivers are limited. This cross-sectional study included 559 children (<16 years) with atopic dermatitis and their caregivers. Disease severity was associated with infants' HRQOL (moderate: IRR: 1.42, 95% CI 1.20-1.67; severe: IRR: 1.72, 95% CI 1.32-2.24). Age and disease severity were associated with children's HRQOL (age: IRR: 0.99, 95% CI 0.98-1.00; moderate: IRR: 1.08, 95% CI 1.02-1.14). Quality of life subdomains itching/scratching, emotional distress and sleep disturbance were most reported and increased with higher disease severity. Both caregivers' mental and physical health were negatively affected by children's HRQOL (physical: IRR: 0.99, 95% CI 0.99-1.00; mental: IRR: 0.98, 95% CI 0.97-0.99). Sociodemographic characteristics (gender, ethnicity, educational attainment of carers, number of children) did not demonstrate significance in children's HRQOL model. In conclusion, current atopic dermatitis diagnostics and treatment have to be extended to the factors influencing both children' as their caregivers' quality of life and adapting management accordingly. Itching/scratching, emotional distress and sleep disturbance deserve attention. Sociodemographic characteristics in children's HRQOL models also merit attention in further research.

Serial outcomes in acute necrotising encephalopathy of childhood: A medium and long term study.

OBJECTIVE: Acute necrotising encephalopathy (ANEC) is a severe, debilitating childhood disorder. We used the ANEC scoring system (ANE-ss) and standardised neurodevelopmental scores to objectively characterise medium and long term outcomes. METHODS: Retrospective review of children with ANEC at KK Women's and Children's Hospital, Singapore, from 2005 to 2012. ANE-ss was determined from clinical features and neuroimaging, and neurodevelopmental scores (Pediatric Glasgow Outcome Scale Extended, Pediatric Cerebral Performance Category scale and Pediatric Overall Performance Category scale) were applied at 1, 6, 12 and 24months post diagnosis. RESULTS: Seven patients with ANEC were studied. All had a viral prodrome with fever, and encephalopathy at presentation, and received immunotherapy (steroids or immunoglobulin). ANE-ss scores were medium risk in 4 patients and high risk in 3 patients. One died (high risk ANE-ss) and outcome was determined in the 6 survivors. At 1month post diagnosis, 3 patients (50%) were mildly affected and 3 (50%) were severely affected. Morbidity rates improved by 12months, with 67% and 33.3% scoring in the mildly affected and severely affected ranges, respectively. Medium risk patients did well with majority having little or no neurological deficits and good outcome scores. CONCLUSION: Mortality and severe morbidity correlated well with high risk ANE-ss. However, our patients with medium risk ANE-ss had good neurodevelopmental sequelae. Serial disability scoring is useful in evaluating the progress of ANEC patients on follow up. Assessment at 1month post diagnosis can aid prognostication of long term outcome.